scholarly journals Clinical relevance of different WHO grade 3 pancreatic neuroendocrine neoplasms based on morphology

2018 ◽  
Vol 7 (2) ◽  
pp. 355-363 ◽  
Author(s):  
Xu Han ◽  
Xuefeng Xu ◽  
Hongyun Ma ◽  
Yuan Ji ◽  
Dansong Wang ◽  
...  
Keyword(s):  
Vascular Invasion ◽  
Who Classification ◽  
Univariate Analysis ◽  
Tnm Staging ◽  
Mitotic Count ◽  
Biological Behavior ◽  
Neuroendocrine Neoplasms ◽  
Ki 67 ◽  
Who Grade ◽  
Pancreatic Neuroendocrine Neoplasms

Purpose Emerging evidence suggests G3 pancreatic neuroendocrine neoplasms (pNENs) present heterogeneous morphology and biology. The 2017 WHO classification has introduced a new category of well-differentiated pancreatic neuroendocrine tumors (WD-pNETs) G3, compared with poorly differentiated pancreatic neuroendocrine carcinomas (PD-pNECs) G3. We aim to analysis the demographics and outcomes of patients with resectable 2017 WHO G3 pNENs to facilitate the distinction between two entities. Methods The multi-institutional retrospective cohort involving 57 surgically treated patients affected by 2017 WHO G3 pNENs were morphologically identified and clinically analyzed. Patients having WD-pNETs G3 and those having PD-pNECs G3 were compared. Results Thirty patients had WD-pNETs and 27 patients had PD-pNECs. The distributions of Ki-67 and mitotic count in patients with PD-pNECs or WD-pNETs showed remarkable disparities. ROC indicated cut-off value of Ki-67 was 45. PD-pNECs were more common in patients with elevated Ki-67 and mitotic count, advanced AJCC TNM stage, vascular invasion, regional lymph-node metastases, elevated NSE and decreased CgA levels compared with WD-pNETs (P < 0.05). The association between 2017 WHO G3 grade and TTR was statistically significant (P < 0.05). Univariate analysis indicated OS rates were associated with morphologic differentiation (WD-pNETs vs PD-pNECs), Ki-67, TNM staging, synchronous distant metastases, initial treatments, vascular invasion, regional lymph nodes metastases, mitotic count and age (P < 0.05). Multivariate analyses illustrated Ki-67, differentiation, TNM staging and vascular invasion were independent predictors (P < 0.05). Conclusions PD-pNECs G3 presented malignant biological behavior and dismal outcome compared with WD-pNETs G3. These findings challenge 2010 WHO classification and suggest the categorization can be improved by refined tumor grading.

scholarly journals Clinicopathological Characteristics of Extrahepatic Biliary Neuroendocrine Neoplasms in the Gallbladder, Extrahepatic Biliary Tract, and Ampulla of Vater: A Single-Center Cross-Sectional Study

2022 ◽  
Author(s):  
Young Mok Park ◽  
Hyung Il Seo ◽  
Byeong Gwan Noh ◽  
Suk Kim ◽  
Seung Baek Hong ◽  
...  
Keyword(s):  
Univariate Analysis ◽  
Survival Rates ◽  
Ampulla Of Vater ◽  
Clinicopathological Characteristics ◽  
Mitotic Count ◽  
Neuroendocrine Neoplasms ◽  
Clinical Report ◽  
Ki 67 ◽  
Poorly Differentiated Neuroendocrine Carcinoma ◽  
Staging Systems

Abstract BackgroundSignificant changes were made in the grading and staging systems for neuroendocrine neoplasms (NENs) in 2017. Therefore, a clinical report to comprehensively update the clinicopathological characteristics, therapeutic approaches, and prognosis of these patients should be drafted.MethodsWe retrospectively reviewed a database of 16 patients who developed NENs or mixed endocrine non-endocrine neoplasms (MiNENs) after curative resection. Among them, eight had ampulla of Vater (AoV) tumors, and eight had non-AoV tumors. One patient had NEN Grade 1, five had NEN Grade 2, and five had NEN Grade 3 (G3); all had poorly differentiated neuroendocrine carcinoma. Five patients had MiNEN or combined carcinoma.ResultsThe mean age was 57.88 years. The overall survival rates after curative surgery at 1, 3, and 5 years were 87.1, 80.4, and 71.4%, respectively. The 1-, 3-, and 5-year disease-free survival rates were 87.1%, 79.8%, and 71.8%, respectively. In univariate analysis, age >65 years, mitotic count (>20/10 high-power fields), Ki-67 index >20%, and presence of stones were significant prognosticators, whereas only the mitotic count was statistically significant in the multivariate analysis. Age >65 years (p=0.039), a Ki-67 index >20% (p=0.03), presence of stones (p=0.021), G3 stage disease, MiNEN (p=0.002), or perineural invasion (p<0.001) were more frequently observed in the non-AoV group than in the AoV group.ConclusionHigh mitosis count had a greater effect on extrahepatic biliary NEN than the tumor-node-metastasis staging system. Patients with AoV tumors had better prognostic predictor factors compared to those with non-AoV tumors.

Multifocal Pancreatic Neuroendocrine Neoplasms In Tuberous Sclerosis: A Rare Case

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S77-S77
Author(s):  
N C Jadhav ◽  
D L Gang
Keyword(s):  
Tuberous Sclerosis ◽  
Neurofibromatosis Type ◽  
Abdominal Ultrasound ◽  
Biologically Active ◽  
Pancreatic Tumors ◽  
Neuroendocrine Neoplasms ◽  
Ki 67 ◽  
Pancreatic Neuroendocrine Neoplasms ◽  
Von Hippel Lindau ◽  
Multifocal Disease

Abstract Casestudy: Pancreatic neuroendocrine neoplasms (PanNEN) are rare accounting for 2-5% of pancreatic tumors. Although mostly sporadic, 10-20% are associated with inherited syndromes, notably MEN-1, Von Hippel- Lindau disease, neurofibromatosis type 1, and tuberous sclerosis (TS). When compared to sporadic cases, PanNEN in hereditary syndromes occur at a younger age, are often multifocal, cystic, and may show characteristic microscopic patterns. TS is an autosomal dominant multi-system disorder with mutations involving TSC1 or TSC2 genes which function as tumor suppressors by inhibiting mTORC1 kinase. PanNEN is observed in 1.5-1.8% of patients with TS and no surveillance guidelines for the assessment of pancreatic lesions are established. Compared to other syndromes, PanNEN associated with TS are solitary. To our knowledge, only two cases of multifocal PanNEN in TS patients have been reported. We present a case of a 67-year-old gentleman with a history of TS also affecting two daughters. He presented to the emergency department with severe abdominal pain. Abdominal ultrasound suggested acute appendicitis and an incidental 2.0 cm solid lesion was noted in the head of the pancreas. Follow-up MRI revealed two additional non-cystic masses in the pancreatic tail. Endoscopic ultrasound-guided biopsy of a tail lesion revealed monomorphic tumor cells with stippled chromatin without cytologic atypia. Immunohistochemical staining was positive for synaptophysin and chromogranin. Ki-67 labelling index was under 1%. Diagnosis of a well-differentiated neuroendocrine tumor (G1) was made. The patient denied symptoms of the carcinoid syndrome and no biologically active hormones were detected. Gallium PET scan revealed multiple foci of radiotracer uptake throughout the pancreas in addition to those described on MRI. Although PanNEN are rare in TS, malignant behavior has been reported. This case reinforces the importance of early detection through active surveillance, especially as surgical options may be limited in multifocal disease.

scholarly journals Clinicopathological heterogeneity between primary and metastatic sites of gastroenteropancreatic neuroendocrine neoplasm

2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Huiying Shi ◽  
Chen Jiang ◽  
Qin Zhang ◽  
Cuihua Qi ◽  
Hailing Yao ◽  
...  
Keyword(s):  
Tumor Metastasis ◽  
Immunohistochemical Analysis ◽  
Metastatic Tumor ◽  
Neuroendocrine Neoplasm ◽  
Neuroendocrine Neoplasms ◽  
Ki 67 ◽  
Who Grade ◽  
Index Variation ◽  
The Difference ◽  
Metastatic Sites

Abstract Background Chromogranin A (CgA), synaptophysin (Syn) and the Ki-67 index play significant roles in diagnosis or the evaluation of the proliferative activity of gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs). However, little is known about whether these biological markers change during tumor metastasis and whether such changes have effect on prognosis. Methods We analyzed 35 specimens of both primary and metastatic tumor from 779 patients who had been diagnosed as GEP-NENs at Wuhan Union Hospital from August 2011 to October 2019. The heterogeneity of CgA, Syn and Ki-67 index was evaluated by immunohistochemical analysis. Results Among these 779 patients, the three most common sites of NENs in the digestive tract were the pancreas, rectum and stomach. Metastases were found in 311 (39.9%) patients. Among the 35 patients with both primary and metastatic pathological specimens, differences in the Ki-67 level were detected in 54.3% of the patients, while 37.1% showed a difference in CgA and only 11.4% showed a difference in Syn. Importantly, due to the difference in the Ki-67 index between primary and metastatic lesions, the WHO grade was changed in 8.6% of the patients. In addition, a Kaplan–Meier survival analysis showed that patients with Ki-67 index variation had a shorter overall survival (p = 0.0346), while neither Syn variation nor CgA variation was related to patient survival (p = 0.7194, p = 0.4829). Conclusions Our data indicate that primary and metastatic sites of GEP-NENs may exhibit pathological heterogeneity. Ki-67 index variation is closely related to the poor prognosis of patients with tumor metastasis, but neither Syn variation nor CgA variation is related to patient prognosis. Therefore, clinicopathologic evaluation of the primary tumor and metastatic sites could be helpful for predicting the prognosis.

scholarly journals Imaging of Pancreatic Neuroendocrine Neoplasms

2021 ◽  
Vol 18 (17) ◽  
pp. 8895
Author(s):  
Giuditta Chiti ◽  
Giulia Grazzini ◽  
Diletta Cozzi ◽  
Ginevra Danti ◽  
Benedetta Matteuzzi ◽  
...  
Keyword(s):  
Functional Imaging ◽  
Imaging Techniques ◽  
Therapy Monitoring ◽  
Somatostatin Receptor ◽  
World Health ◽  
Biological Behavior ◽  
Pancreatic Tumors ◽  
Neuroendocrine Neoplasms ◽  
Imaging Features ◽  
Pancreatic Neuroendocrine Neoplasms

Pancreatic neuroendocrine neoplasms (panNENs) represent the second most common pancreatic tumors. They are a heterogeneous group of neoplasms with varying clinical expression and biological behavior, from indolent to aggressive ones. PanNENs can be functioning or non-functioning in accordance with their ability or not to produce metabolically active hormones. They are histopathologically classified according to the 2017 World Health Organization (WHO) classification system. Although the final diagnosis of neuroendocrine tumor relies on histologic examination of biopsy or surgical specimens, both morphologic and functional imaging are crucial for patient care. Morphologic imaging with ultrasonography (US), computed tomography (CT) and magnetic resonance imaging (MRI) is used for initial evaluation and staging of disease, as well as surveillance and therapy monitoring. Functional imaging techniques with somatostatin receptor scintigraphy (SRS) and positron emission tomography (PET) are used for functional and metabolic assessment that is helpful for therapy management and post-therapeutic re-staging. This article reviews the morphological and functional imaging modalities now available and the imaging features of panNENs. Finally, future imaging challenges, such as radiomics analysis, are illustrated.

Textural analysis on contrast-enhanced CT in pancreatic neuroendocrine neoplasms: association with WHO grade

2018 ◽  
Vol 44 (2) ◽  
pp. 576-585 ◽  
Author(s):  
Chuangen Guo ◽  
Xiaoling Zhuge ◽  
Zhongqiu Wang ◽  
Qidong Wang ◽  
Ke Sun ◽  
...  
Keyword(s):  
Textural Analysis ◽  
Neuroendocrine Neoplasms ◽  
Who Grade ◽  
Pancreatic Neuroendocrine Neoplasms ◽  
Contrast Enhanced Ct ◽  
Contrast Enhanced ◽  
Enhanced Ct

Loco-regional outcomes of a population-based cohort of rectal neuroendocrine tumors.

2016 ◽  
Vol 34 (4_suppl) ◽  
pp. 668-668
Author(s):  
Erica S Tsang ◽  
Yarrow Jean McConnell ◽  
David F. Schaeffer ◽  
Caroline Speers ◽  
Hagen F. Kennecke
Keyword(s):  
Surgical Resection ◽  
Neuroendocrine Tumors ◽  
Local Excision ◽  
Univariate Analysis ◽  
Local Relapse ◽  
Population Based ◽  
Mitotic Count ◽  
Margin Status ◽  
Ki 67 ◽  
Pelvic Radiation

668 Background: Optimal management of rectal neuroendocrine tumors (NETs) is not well defined. We characterized the clinicopathologic features, loco-regional, and systemic management of a population-based cohort of rectal NETs. Methods: Patients diagnosed with rectal NETs from 1999-2011 were identified from British Columbia provincial databases. NETs were classified as G1 and G2 tumors with a Ki-67 ≤ 20% and/or mitotic count ≤ 20 per high power field. Results: Of 91 rectal NETs, median age was 58 (IQR 48-65) years and 35 (38%) were male. Median tumor size was 6 (IQR 4-8) mm. Median overall survival was 164.7 months, with 3 patients presenting with stage IV disease. Treatment included local excision (n = 79), surgical resection (n = 6), and pelvic radiation (n = 1; T3N1 tumor). Final margin status was positive in 17 (20%) cases. Local relapse occurred in 8 (9%) cases, and one relapse to bone 13 months after T3N1 tumor resection. Univariate analysis demonstrated an association between local relapse and T classification, Ki-67, mitotic count, grade, and perineural invasion (p< 0.01), but not N or M classification, or lymphovascular invasion. Local relapse was not associated with surgical management or margin status. Of 3 patients with metastatic disease, two received systemic management, with capecitabine and temozolomide. Conclusions: Rectal NETs generally presented with small, early tumors and were treated with local excision or surgical resection without pelvic radiation. [Table: see text]

scholarly journals Activity and Safety of Standard and Prolonged Capecitabine/Temozolomide Administration in Patients with Advanced Neuroendocrine Neoplasms

2019 ◽  
Vol 109 (4) ◽  
pp. 333-345 ◽  
Author(s):  
Eleftherios Chatzellis ◽  
Anna Angelousi ◽  
Kosmas Daskalakis ◽  
Marina Tsoli ◽  
Krystallenia I. Alexandraki ◽  
...  
Keyword(s):  
Univariate Analysis ◽  
Area Under The Curve ◽  
Progression Free Survival ◽  
Clinicopathological Characteristics ◽  
Unknown Primary ◽  
Neuroendocrine Neoplasms ◽  
Significant Prognostic Factor ◽  
Characteristic Analysis ◽  
Prolonged Administration ◽  
Ki 67

Background: Capecitabine and temozolomide combination (CAPTEM) is associated with high response rates in patients with advanced neuroendocrine neoplasms (NENs). We evaluated the real-world activity and safety of CAPTEM from 3 NEN centers. Methods: Clinicopathological characteristics and outcomes of patients treated with CAPTEM for bulky or progressive disease (PD) were retrospectively analyzed. ­Results: Seventy-nine patients with gastroenteropancreatic (grades 1–2 [n = 38], grade 3 [n = 24]) and lung/thymic (n = 17) NENs were included. Median treatment duration was 12.1 months (range 0.6–55.6). Overall, partial responses (PRs) occurred in 23 (29.1%), stable (SD) in 24 (30.4%), and PD in 28 (35.4%) patients. Median progression-free survival (PFS) and overall survival (OS) were 10.1 (6–14.2) and 102.9 months (43.3–162.5), respectively. On univariate analysis, NENs naive to chemotherapy and low Ki67 were associated with favorable responses (partial response [PR] + SD; p = 0.011 and 0.045), PFS (p < 0.0001 and 0.002) and OS (p = 0.005 and 0.001). Primary site (pancreas and lung/thymus) was also a significant prognostic factor for PFS (p < 0.0001) and OS (p < 0.0001). On multivariate analysis, gastrointestinal and unknown primary NENs (hazard ratio [HR] 0.3, 95% CI 0.1–0.8, p = 0.009 and p = 0.018) and prior surgery (HR 2.4, 95% CI 11–4.9, p = 0.021) were independent prognostic factors for PFS. Ki-67 was a poor predictor for favorable response in receiver operating characteristic analysis (area under the curve 0.678). Safety analysis of CAPTEM indicated rare events of serious (grades 3–4) toxicities (n = 4) and low discontinuation rates (n = 8) even in patients with prolonged administration (>12 months). Conclusions: CAPTEM treatment can be an effective and safe treatment even after prolonged administration for patients with NENs of various sites and Ki67 labeling index, associated with significant favorable responses and PFS.

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